Vasculitis Program

Penn Rheumatology is recognized as a leader in the treatment of vasculitis. The division consists of expert clinicians and researchers whose mission is to provide the most progressive therapies available in the world.

What is Vasculitis?

Vasculitis is an autoimmune disorder that causes inflammation of the blood vessels. For reasons that are unknown, the body's immune system attacks the blood vessels, causing inflammation. When inflammation occurs, the walls of the blood vessels thicken and narrow, reducing the amount of blood and oxygen delivered to vital organs. In some cases, a blood vessel will weaken, forming a bulge or aneurysm which can be life threatening. Any of the body's blood vessels can be affected including arteries, veins and capillaries.

Vasculitis can cause serious complications depending on which organs and blood vessels are affected. There are many different types of vasculitis. A patient's prognosis depends greatly on the type of vasculitis and how quickly the condition progresses.

Types of Vasculitis

There are many different types of vasculitis:

Behçet's disease — Can occur in blood vessels of any size and commonly affects the mouth, eyes and genitals.

Buerger's disease — Typically affects blood flow to the extremities and is commonly found in smokers.

Churg Strauss syndrome (CSS) — Small to medium sized blood vessels are affected. Symptoms may include asthma, granulomas (abnormal lumps) and a high level of white blood cells. Organs and body tissues such as the lungs, heart, skin, digestive system, kidneys, joints and sinuses can be affected.

Cryoglobulinemia — Linked to hepatitis C and lymphoma. Common symptoms include nerve damage, rash on lower extremities and arthritis.

Giant cell arteritis (GCA) — Also known as temporal arteritis, GCA most commonly affects Caucasian women and those over age 50. Symptoms include fever, loss of vision, headaches and pain in the shoulders, hips and jaw.

Henoch–Schönlein purpura (HSP)— Affects the stomach, kidneys, joints and skin. HSP is commonly found in children following an upper respiratory infection and is generally not severe.

Kawasaki disease — A rare form of vasculitis affecting mostly children under age five. Symptoms include high fever; swollen hands and feet; red lips, eyes, and mouth; rash and swollen lymph nodes.

Microscopic polyangiitis (MPA) — Can affect any organ in the body, but is most commonly found in the skin, kidneys and nerves.

Polyarteritis nodosa (PAN) — Can be triggered by hepatitis B or a form of leukemia called hairy cell leukemia. Men are more likely to get PAN than women. PAN can be found in any organ, but most commonly affects the kidneys, skin and nerves.

Polymyalgia rheumatica (PMR) — Associated with giant cell arteritis and can affect the shoulders and hips.

Rheumatoid vasculitis (RV) — Commonly found in patients with rheumatoid arthritis, RV affects many different organs.

Takayasu's arteritis (TA) — Affects the aorta and the branches that carry blood away from the heart.

Causes of Vasculitis

The exact cause of vasculitis is unknown, but certain factors may trigger the disease:

Infections, such as Hepatitis B or Hepatitis C

Allergic reactions to certain medications

Autoimmune diseases

Types of cancers that affect the blood, such as leukemia and lymphoma

Diagnosing Vasculitis

The following tests are performed to diagnose vasculitis:

Blood test — Reveals signs of inflammation and certain antibodies that correlate with immune system disorders.

Urine test — Checks for abnormalities that can indicate a problem, such as red blood cells or protein in the urine.

Angiogram — A type of X–ray performed using a straw–like catheter inserted into larger veins or arteries. A special dye is injected that reveals inflammation on the X–ray.

Biopsy — A piece of tissue from affected arteries, veins or organs is examined for signs of vasculitis.

Treating Vasculitis

Depending upon the type and severity, treatment options for vasculitis include certain medications to control the disease. Medications may be prescribed to lessen inflammation and suppress the immune system:

Corticosteroid medication

Immunosuppressive medication

If organs such as the heart, lungs or kidneys are affected, other treatment options may be considered to improve the function of these organs.

As a recognized leader in the management of vasculitis, the Penn Rheumatology team is skilled at providing all forms of treatment options.